A typical case of progeria (Hutchinson-Gilford syndrome) is reported including the findings of the necropsy. Death occurred at the age of 11 years as the result of an accident. Analysis of cephalometric roentgenograms of this patient revealed that the characteristic facies of progeria results from marked retardation of facial growth in the presence of relatively normal growth of the neurocranium, and is therefore not the result of premature aging. Atherosclerosis was found in this child at necropsy. Concentrations of lipoproteins in the serum determined by analytical ultracentrifugation during life were found to be abnormally high, suggesting a metabolic error rather than premature aging of the arteries as the cause of the atherosclerosis. Psychologic studies showed the patient to be of normal intelligence with no evidence of senilism. Analysis of these data leads to the conclusion that premature aging does not occur in progeria.
Psychobogic tests were performed with five female pseudohermaphrodites with congenital adrenal hyperplasia both before and some time after clitoridectomy. Of these children, four had been reared as girls. The child who had been reared as a boy underwent change of sex assignment at the age of 6 years, after performance of the initial psychologic tests. None of the children showed evidence of significant psychopathology before clitoridectomy. None showed evidence of deleterious psychologic effects after the operation. The child whose sex was changed at the age of 6 years apparently made a good adjustment and showed no adverse effects as evaluated by psychologic tests.
If there is no significant psychopathology, clitoridectomy may be performed without fear of deleterious psychologic effects in female pseudohermaphrodites who have been reared as girls. While, in general, change of sex assignment should not be done in any hermaphrodite beyond the infantile age period, female pseudohermaphrodites with adrenogenital syndrome who have been reared as boys, because of an original diagnostic error, should be individually evaluated with regard to possible change of sex assignment. Despite the apparent success in the case reported here with its unusual special circumstances, the psychologic hazards involved will make reassignment of sex inadvisable in most of these cases.
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